Object: Neurofibromatosis Type 2 (NF2) has typically resulted in deafness after surgical removal of bilateral vestibular schwannomas (VSs). Cochlear implants are generally ineffective for this kind of deafness because of the loss of continuity in the auditory nerve after tumor removal. The first auditory brainstem implant (ABI) in such a patient was performed in 1979 at the House Ear Institute, and this individual continues to benefit from electrical stimulation of the cochlear nucleus complex. In 1992, an advanced multichannel ABI was developed and a series of patients with NF2 received this implant to study the safety and efficacy of the device.
Methods: At the time of first- or second-side VS removal, patients received an eight-electrode array applied to the surface of the cochlear nucleus within the confines of the lateral recess of the fourth ventricle. The device was activated approximately 6 weeks after implantation. and patients were tested every 3 months for the 1st year after the initial stimulation, and annually thereafter. The protocol included a comprehensive battery of psychophysical and speech perception tests.
Conclusions: The multichannel ABI proved to be effective and safe in providing useful auditory sensations in most patients with NF2. The ABI improved patients' ability to communicate compared with the lipreading-only condition, it allowed the detection and recognition of many environmental sounds, and in some cases it provided significant ability to understand speech by using just the sound from the ABI (with no lipreading cues). Its performance in most patients has continued to improve for up to 8 years after implantation.